Similar content of phospholipids and gangliosides in normal and homozygous familial hypercholesterolemia fibroblasts

P. H. Fishman, R. M. Bradley, M. S. Brown, J. R. Faust, J. L. Goldstein

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The cellular content of total and individual phospholipids and gangliosides was measured in fibroblasts cultured from four normal subjects, three patients with lysosomal lipid storage diseases, and two subjects with homozygous familial hypercholesterolemia. Measurements were made on cells grown in medium containing fetal calf serum under conditions in which normal cells derive cholesterol for cell growth from low density lipoprotein present in the fetal calf serum, whereas familial hypercholesterolemia homozygote cells, which lack cell surface low density lipoprotein receptors, derive cholesterol from endogenous synthesis. No difference was observed in the cellular content of total or individual phospholipids and gangliosides in the normal and familial hypercholesterolemia homozygote cells. In contrast cells from a patient with Niemann-Pick disease and a patient with Sandhoff disease showed elevations in the content of sphingomyelin and complex gangliosides, respectively.

Original languageEnglish (US)
Pages (from-to)304-308
Number of pages5
JournalJournal of lipid research
Issue number3
StatePublished - Nov 24 1978


ASJC Scopus subject areas

  • Biochemistry
  • Endocrinology
  • Cell Biology

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